Kawasaki Disease In Children: Symptoms, Causes & Treatment

The disease has several underlying complex causative factors, and treatment must be prompt.

Medically reviewed by Dr. Dur Afshar Agha, MS Dr. Dur Afshar Agha Dr. Dur Afshar AghaMS facebook_iconinsta_icon Specialty: Masters of Tropical PediatricsExperience: 26 years
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Kawasaki disease in children is also called Kawasaki syndrome or mucocutaneous lymph node. It is most common in children below five years and shows acute fever-like symptoms (1). Characterized by swelling and inflammation of blood vessels, the non-contagious Kawasaki disease can lead to certain complications, such as an aneurysm (2).

Although rare, the disease accounts for the leading cause of acquired heart disease in young children and babies in the US (3). However, children suffering from it can recover from timely diagnosis and treatment.

This post will help you understand Kawasaki disease in children, including its causes, symptoms, and treatments.

Causes And Risk Factors For Kawasaki Disease In Children

Kawasaki Disease (KD) is a disease with no known cause but is believed to result from an autoimmune reaction (3). Some of the risk factors that increase a child’s chances of developing Kawasaki disease include (4)

  • Age: Kawasaki disease generally affects children below five years of age. Two years is the average age when children develop the condition.
  • Gender: Boys are 1.5 times more likely to develop the condition than girls.
  • Ethnicity: Kawasaki disease can affect children of any ethnicity. However, it is more common in Japanese children and those of Asian and Pacific Island ancestry.

Symptoms Of Kawasaki Disease In Children

Kawasaki disease usually begins with a fever above 102°F (38°C) that lasts at least five days. The other symptoms and clinical features of KD that follow the fever may include (1) (3)

  • Body rash, which is particularly severe in the diaper area.
  • Red, inflamed eyes without pus, drainage, or crusting.
  • Swollen lymph nodes on one side of the neck.
  • Swollen hands and feet with redness on the palms and the soles.
  • Excessively red, swollen, and cracked lips.
  • Strawberry-like dark-pinkish tongue with rough red spots.
  • Extreme irritability and fussiness.
  • Peeling of the skin on the fingers and toes, typically beginning two to three weeks after the fever develops.

All of these symptoms may not be present in a child at the same time. Also, some other general symptoms that a child may manifest are

Most of the symptoms of Kawasaki disease are non-specific, so do not self-diagnose. Instead, consult a doctor promptly if your child has a fever and any of the symptoms mentioned above.

Diagnosis Of Kawasaki Disease

There’s no specific test to diagnose Kawasaki disease. Usually, the doctor does a physical examination of the child and notes their medical and family history. If the physical exam suggests the child has Kawasaki disease, immediate hospitalization is warranted, and the following tests may be conducted (2) (5).

  1. Blood and urine tests: The blood test checks the blood profile and looks for an abnormal amount of blood cells. On the other hand, the urine test helps examine the urine for proteins that indicate inflammation.
  2. Electrocardiogram (EKG): This test examines the heart’s electrical activity to determine abnormal heart rhythm and structure. The doctor may also order an echocardiogram (ECG) to locate any abnormality in the heart muscle and valves.
  3. Cardiac catheterization or coronary angiogram: It’s an invasive test in which a small tube is inserted into the blood vessels to observe the coronary arteries using contrast and X-ray.

Depending on the test results, the doctor may refer you to a pediatric specialist for appropriate guidance about diagnosis and treatment.

Treatment For Kawasaki Disease

A child diagnosed with KD is immediately admitted to the hospital, where they are administered intravenous gamma globulin (IVIG) through a vein for over eight to 12 hours. IVIG provides extra antibodies that help fight infection and inflammation. The child is then kept under observation for at least 24 hours to ensure the symptoms improve and the fever subsides (3).

A repeat dose of IVIG or other medications may be given if the fever returns or the first ECG report indicates an issue. Steroids, infliximab, or etanercept are some medications that the doctor may prescribe to reduce inflammation.

Aspirin in moderate doses may also be prescribed to alleviate heart problem risks. The doctor may also seek the opinion of pediatric specialists in cardiology, rheumatology, or infectious disease in such conditions.

Note: Aspirin is a non-steroidal anti-inflammatory drug (NSAID) that young children should be given only under a healthcare provider’s guidance. Unguided use of aspirin can cause a serious liver condition called Reye Syndrome.

Follow-up Care

Children discharged from the hospital are prescribed a low-dose aspirin that they need to take every day for six to eight weeks. During this time, the child may feel extreme fatigue, and the skin on their hands and feet may peel off. These signs aren’t a cause for concern. However, call your healthcare provider if the child develops a fever or any of the symptoms of KD reappear.

Also, aneurysms may develop after the first couple of weeks of illness. So, close monitoring of children recovering from KD is vital. This is precisely why children are scheduled for a check-up at two weeks and then at six to eight weeks post the onset of fever. If any abnormality is detected, the doctor may refer you to a specialist, and more frequent check-ups would be needed.

Note: Postpone live viral vaccines, such as MMR (measles, mumps, rubella) and Varicella (chickenpox), for at least 11 months after your child has been treated with IVIG. It’s necessary as IVIG can reduce the efficacy of vaccines.

Complications Of Kawasaki Disease

If not treated promptly, KD can lead to adverse complications, such as inflammation of the coronary arteries. Coronary arteries are the blood vessels that supply oxygen-rich blood to the heart. Chronic inflammation of the coronary arteries could lead to coronary artery aneurysm, a condition wherein the weakened and damaged blood vessel walls bulge out like a balloon (3).

The other complications may include (5)

  • Inflammation of the heart muscle (myocarditis), heart lining (endocarditis), or heart covering (pericarditis).
  • Improper functioning of the heart valves.
  • Heart failure (rare case).

Besides these, KD may also affect the nervous, immune, digestive, and urinary systems. Thus, timely diagnosis and prompt treatment of KD are crucial. According to experts, the risk of coronary aneurysms reduces if the treatment begins within the first ten days of the illness (3).

Thus, children without aneurysms can recover from the condition well and maintain long-term health by following a heart-healthy diet and doing regular exercise. However, children with aneurysms require continuous monitoring and special long-term care by a pediatric cardiologist.

Frequently Asked Questions

1. What is the survival rate of Kawasaki disease?

With effective and timely treatment, the future outlook for Kawasaki disease looks good, with around an 88 to 95% survival rate (6).

2. What are the stages of Kawasaki disease?

Kawasaki disease has three clinical phases: acute, sub-acute, and convalescent. The acute phase lasts 14 days and ends with a fever, followed by the sub-acute phase ending on day 25. The last phase, convalescence, begins after this and ends in the sixth or eighth week of the disease (7).

Kawasaki disease is a febrile illness that has a sudden onset and no identifiable causes. Fever above 102°F (38°C) is the most common symptom of KD, which can be followed by other non-specific symptoms, such as nausea, stomach cramps, and body rash. Since KD can cause inflammation to the coronary arteries and lead to aneurysms, timely diagnosis and treatment are crucial. If you suspect your child has KD, seek immediate medical assistance.

Key Pointers

  • Kawasaki disease in children is characterized by inflamed blood vessels.
  • Although the cause of this disease is unknown, it is believed to be an autoimmune reaction.
  • Fever above 102°F, body rash, inflamed eyes, and swollen lymph nodes are some common symptoms of Kawasaki disease.
  • Administration of intravenous gamma globulin is a common treatment option for this condition.

References

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Dr. Dur Afshar Agha is a consultant pediatrician with around 26 years of experience in various medical facilities both in Pakistan and Saudi Arabia. She has headed the Department of Preventive Pediatrics at the prestigious, Children’s Hospital and Institute of Child Health in Pakistan and is a life member of the Pakistan Paediatric Association.

Read full bio of Dr. Dur Afshar Agha
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